Tracheo-Esophageal Fistula · • Esophageal dysmotility -domperidone • Tracheomalacia...
Transcript of Tracheo-Esophageal Fistula · • Esophageal dysmotility -domperidone • Tracheomalacia...
Tracheo-Esophageal Tracheo Esophageal Fistula
Uri Greenwald, MDDownstate Medical CenterDivision of Pediatric SurgeryOctober 12th, 2007
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PresentationPresentation• xx weeker born NSVD.
APGAR 9 d 9 2 435APGAR 9 and 9. 2,435g.• Prenatal US showed dilated
coronar sin s hich coronary sinus which prompted planned NICU admission for admission for cardiopulmonary monitoring. g
• Baby immeadiately noted to have retractions
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PresentationPresentationHEENT: WNL. 18 7
19.2230
138 104 6 64HEENT: WNL.CV: RRR, s1, s2.Chest: Good air entry b/l
18.756.2 5.1 23 0.6
Chest: Good air entry b/l.Abd: soft nontender,
nondistened +bowel nondistened. bowel sounds.
Ext: FROM. No deformaties.: O . o de o a es.GU: Normal male.Back: WNLBack: WNL.
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PresentationPresentation• DOL#1 Placed on CPAP. DOL#1 Placed on CPAP.
NPO • DOL #2 TTE showed O s o ed
mesocardia with bilateral SVC. Left sided aortic arch.
• DOL #2 OGT was tt t d d ld t attempted and would not
pass.
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OROR• Chest entered via 5th
intercostal space.• Extrapleural Approach. • Azygous vein divided. • TEF fistula ligated.• Esophageal pouch freed.
Opening made and single layer anastamosis between layer anastamosis between upper esophageal pouch and lower esophagus.
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Post opPost op• POD #4 ExtubatedPOD #4 Extubated• POD #5 Feeds via OGT• POD #6 PO feeds• POD #6 PO feeds• POD #7 CT removed
inadvertanly inadvertanly • POD #8 Tolerating PO,
+BM, cleared for D/C , c ea ed o /C home by Pediatric Surgery service
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EpidemiologyCongenital EA: 1 in 2000 4000 Congenital EA: 1 in 2000 - 4000 live births. Highest incidence in Finland 1 in 2500.
25-40% premature or low birthweight
50% have associated defects. Cardiac 35% GI 24% GU 20% Cardiac 35%, GI 24%, GU 20%, Vertebral and Radial 15% VACTERL.
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Hi t History Thomas Gibson 1697The Anatomy of the Humane Bodies EpitomizedThe Anatomy of the Humane Bodies Epitomized
Harry Richter 1913Transpleural approach. Ligation and gastrostomya sp eu a app oac . ga o a d gas os o y
Thomas Lanman 1936Extrapleural approach. Ligation and anastamosis
William Ladd 1939Extrapleural 4 stage
Cameron Haight 1941Extrapleural. Ligation and anastamosisJae-O. Bae, et al. Cameron Haight: Pioneer in the Treatment of Esophageal Atresia. Current Surgery. Volume 62, Issue 3 Pages 327-329
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Embr ologEmbryology• Primitive foregut• Week 4-6: Caudal part of the
foregut forms a ventral diverticulum that evolves into t h trachea
• The longitudinal tracheoesophageal fold fuses to form a septum dividing the to form a septum dividing the foregut into a ventral laryngotracheal tube and a dorsal esophagus dorsal esophagus.
• Posterior deviation of the septum causes incomplete separation of the esophagus separation of the esophagus from the laryngotracheal tube
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ScienceScience• MultifactoralMultifactoral• Sonic Hedgehog Signaling
Pathway absenta ay abse• Defective FGF Signaling
pathwayp y• Environmental:
-Contraceptives-MethimazoleMethimazole-Benectin
Spilde, T, et al. Fibroblast Growth Factor Signaling in the Developing Tracheoesophageal Fistula. Journal of Pediatric Surgery, Vol 38, No 3 (March), 2003: pp 474-477
Spilde, T., et al. A Role for Sonic Hedgehog Signaling in the Pathogenesis of HumanTracheoesophageal Fistula. Journal of Pediatric Surgery, Vol 38, No 3 (March), 2003: pp 465-468
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PresentationPresentation• Respiratory Distress (coughing, p y ( g g
choking, regurgitation with first feeding)
• Polyhydramnios: TEF 33%. EA Polyhydramnios: TEF 33%. EA 100%.
• Gastric distentionD t ti ith i li • Desaturation with nippling
• Inability to pass OGT• Radiographs show OGT in Radiographs show OGT in
upper esophagus with air in stomach(except in proximal fistula).fistula).
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PrognosisPrognosis• Category A patients > 5.5 lb (2.5 kg)
t bi th d h th i ll at birth and who are otherwise well • Category B patients 4-5.5 lb (1.8-2.5
kg) and are well or have higher birth weight and moderate pneumonia weight and moderate pneumonia and other congenital anomaly;
• Category C patients < 4 lb (1.8 kg) or have higher birth weight and severe g gpneumonia and severe congenital anomaly.
• Category A(98%), immediate primary i repair
Category B(82%), delayed repair Category C(50%), staged repair
1) Waterston, DJ. et al. Congenital tracheo-oesophageal fistula in association with oesophageal atresia. Lancet. 1963 Jul 13;2:55-7. 2) Lopes PJ, Keys C, Pierro A, et al. Oesophageal atresia: improvedoutcome in high risk groups? J Pediatr Surg 2006;41:331- 4.
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Preoperati elPreoperatively• Head elevationHead elevation• OGT suction• Repeated suctioning• Repeated suctioning• H2 blockers• NPO• NPO• Avoid bag mask venilation
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Preoperati elPreoperatively• Echocardiogramg
-Must assess for Right sided aortic arch. Found in 2.5% of children with EA.-PDA
• Renal US-Bilateral renal agenesis -Multicystic dysplastic kidneys-May need to follow with renal scanMay need to follow with renal scan
• Bronchoscopy -Detecton of upper pouch fistula-Localization of distal fistula-Detection of aberant RUL bronchus
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RepairRepair• Right thoracotomy via Right thoracotomy via
4th/5th intercostal space• Fistula division close to s u a d s o c ose o
trachea• Mobilization of proximal p
esophagus• Feeding tube across
anastamosis• Single layer anastamosis
T i f-Tension free
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Complications EarlComplications Early• Anastomotic leak: 15%. Anastomotic leak: 15%.
- 3 or 4 days post-op. Saliva in chest tube. +/-sepsis
• Anastomotic stricture: 50% Anastomotic stricture: 50%. -Functionally significant?-Balloon dilitation under floroscopic control. 6weeks
old and at least 4weeks post surgery
Blaire, G. Esophageal Atresia With or Without Tracheoesophageal Fistula. Emedicine. Oct 17, 2007.
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Complications LateComplications Late• Gastroesophageal RefluxGastroesophageal Reflux
-Congenital distal dysmotility-Dysfunction of physiologic antireflux barrier-Vagal injury/dysfunction leading to gastric g j y y g g
dysmotility.-H2 blockers post op for 6months-Nissen fundiplication
• Esophageal dysmotility -domperidone
• Tracheomalacia Tracheomalacia -Bronchoscopy reveals trachea that significantly collapses, flattens, or closes on expiration.
Blaire, G. Esophageal Atresia With or Without Tracheoesophageal Fistula. Emedicine. Oct 17, 2007.
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ComplicationsComplications
D.C. Little, et al. Long-Term Analysis of Children With Esophageal Atresia andTracheoesophageal Fistula. Journal of Pediatric Surgery. Vol 38, No 6 (June), 2003: pp 852-856
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ComplicationsComplications
D.C. Little, et al. Long-Term Analysis of Children With Esophageal Atresia andTracheoesophageal Fistula. Journal of Pediatric Surgery. Vol 38, No 6 (June), 2003: pp 852-856
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Long GapLong Gap• A) Gap length exceeding ) p g g
4cm.B) Gap length greater th 2 t b l b dithan 2 vertebral bodies.
• 2-6 vertebral bodies. Delayed repair 8 12 weeksDelayed repair 8-12 weeks.
• >6 vertebral bodies will need to replace eed o ep ace esophagus at 3-4months
Spitz, L. Esophageal atresiaB Lessons I have learned in a 40-year experienceJournal of Pediatric Surgery (2006) 41, 1635–1640
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Treatment/LengtheningTreatment/Lengthening• Gastrostomy. y
-Gapogram.
• BougienageT ti t• Traction sutures
• Myotomy-Between muscularis propria and serosa Between muscularis propria and serosa -1cm
• Stomach mobilization
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